Moyamoya disease: a review of the literature.

Moyamoya disease is a rare progressive cerebrovascular disorder that can be congenital or acquired and involves progressive stenosis and occlusion of cerebral arteries. The brain through compensatory angeogenesis then attempts to compensate for ischemia by producing a local network of tiny blood vessels, which appear cloud-like on angiograms. Consistent with multifocal or diffuse areas of ischemia, people with moyamoya often have multiple areas of cognitive impairment. A thorough literature review of the neuropsychological profile in individuals diagnosed with moyamoya disease is provided. Medical and neuropsychological/neurobehavioral data of a middle-aged woman with moyamoya disease is also described. The patient (MA) suffered an embolic shower with insult to both cerebral hemispheres. Neuropsychological results suggested a multifocal decline, with both cortical and subcortical involvement. Data were consistent with impairments in attention, concentration, executive skills, processing speed, and memory encoding and retrieval, with relatively spared aspects of memory and language skills. This case study supports the literature and provides an additional example of the neuropsychological profile and deficit pattern of an individual with moyamoya following an embolic stroke shower.